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September 14, 2021; 97 (11) Resident & Fellow Section

Teaching Video NeuroImage: Reflex Facilitation in Lambert-Eaton Myasthenic Syndrome

Antonio Edvan Camelo-Filho, View ORCID ProfileEduardo de Paula Estephan, View ORCID ProfileCarlos Otto Heise, View ORCID ProfileEdmar Zanoteli
First published May 26, 2021, DOI: https://doi.org/10.1212/WNL.0000000000012180
Antonio Edvan Camelo-Filho
From the Department of Neurology (A.E.C.-F., E.d.P.E., C.O.H., E.Z.), Faculdade de Medicina da Universidade de São Paulo; and Department of Neurology (E.d.P.E.), Faculdade de Medicina Santa Marcelina, São Paulo, Brazil.
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Eduardo de Paula Estephan
From the Department of Neurology (A.E.C.-F., E.d.P.E., C.O.H., E.Z.), Faculdade de Medicina da Universidade de São Paulo; and Department of Neurology (E.d.P.E.), Faculdade de Medicina Santa Marcelina, São Paulo, Brazil.
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Carlos Otto Heise
From the Department of Neurology (A.E.C.-F., E.d.P.E., C.O.H., E.Z.), Faculdade de Medicina da Universidade de São Paulo; and Department of Neurology (E.d.P.E.), Faculdade de Medicina Santa Marcelina, São Paulo, Brazil.
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Edmar Zanoteli
From the Department of Neurology (A.E.C.-F., E.d.P.E., C.O.H., E.Z.), Faculdade de Medicina da Universidade de São Paulo; and Department of Neurology (E.d.P.E.), Faculdade de Medicina Santa Marcelina, São Paulo, Brazil.
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Teaching Video NeuroImage: Reflex Facilitation in Lambert-Eaton Myasthenic Syndrome
Antonio Edvan Camelo-Filho, Eduardo de Paula Estephan, Carlos Otto Heise, Edmar Zanoteli
Neurology Sep 2021, 97 (11) e1168-e1169; DOI: 10.1212/WNL.0000000000012180

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A 41-year-old woman presented with progressive fluctuating lower limb weakness, urinary incontinence, and dry eyes and mouth. Physical examination revealed proximal flaccid tetraparesis (Medical Research Council 4). The patellar reflex was absent, but with postexercise facilitation (video 1). Eyelid and eye movements were normal. Electroneuromyography revealed a presynaptic neuromuscular junction disorder (figure). Antibodies to anti-voltage-gated calcium channels were positive. Periodic cancer screening, including whole-body FDG-PET, was negative during 2 years of follow-up. These findings were consistent with nonparaneoplastic Lambert-Eaton myasthenic syndrome.1 The patient had a good response to IV immunoglobulin. The presence of ascending muscle weakness, autonomic dysfunction, and postexercise facilitation of reflexes should raise suspicion for the diagnosis.

Video 1

Facilitation of patellar reflex in Lambert-Eaton myasthenic syndrome. The first part of the video shows the patient with absent patellar reflex that was elicited after a sustained muscular contraction, known as postexercise facilitation. In the second part of the video, the patient presents a normoactive patellar reflex after treatment. Absent/reduced reflex, as well as postexercise facilitation of reflex, are specific clinical clues to the diagnosis of Lambert-Eaton myasthenic syndrome.Download Supplementary Video 1 via http://dx.doi.org/10.1212/012180_Video_11

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Figure Neurophysiologic Findings at Abductor Digiti Minimi

(A) Low-frequency repetitive nerve stimulation shows a decremental response (31.6%) at 3 Hz. (B) High-frequency repetitive nerve stimulation shows an incremental response (164%) at 30 Hz. (C) Compound muscle action potential amplitude increment of 400% after a 10-second exercise (postexercise facilitation).

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The authors report no targeted funding.

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The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

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  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • ↵* These authors contributed equally to this work.

  • Teaching slides links.lww.com/WNL/B419

  • © 2021 American Academy of Neurology

Reference

  1. 1.↵
    1. Kesner VG,
    2. Oh SJ,
    3. Dimachkie MM,
    4. Barohn RJ
    . Lambert-Eaton myasthenic syndrome. Neurol Clin. 2018;36(2):379-394.
    OpenUrlPubMed

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